Pediatric Gastroenterology and Hepatology Research

ChiLDREN is a collaborative network organized to study rare childhood liver diseases, sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Children’s is among only 16 pediatric centers in the country to participate in the network.

The goals of ChiLDREN include:

• Facilitating the discovery of causes of childhood liver disease.
• Developing new diagnostics tests or disease markers.
• Developing treatment options for children with liver disease and those who undergo liver transplant.
• Training the next generation of investigators in rare pediatric liver diseases.

Diseases studied by the network include:

• Alagille syndrome
• Alpha 1 antitrypsin deficiency (A-1AT)
• Bile acid synthesis and metabolic defects
• Biliary atresia
• Cystic fibrosis liver disease
• Idiopathic neonatal hepatitis
• Mitochondrial hepatopathies
• Progressive familial intrahepatic cholestasis

Protocols that are part of ChiLDREN include:

• Prospective Database of Infants With Cholestasis (PROBE): A study of infants up to age 6 months with jaundice from liver disease.
• Biliary Atresia Study in Infants and Children (BASIC): A study to gather information on children with biliary atresia to understand what happens after the Kasai surgery.
• Longitudinal Studies in Children With Cholestasis (LOGIC): A study that involves children with alpha-1 antitrypsin deficiency, Alagille syndrome, progressive familial intrahepatic cholestasis and bile acid defects.
• Longitudinal Studies of Mitochondrial Hepatopathies (MITOHEP): A study to gather information about liver problems in mitochondrial diseases and in fatty oxidation defects.

Children’s is the largest enroller of patients in ImproveCareNow, a national outcomes and quality registry for children with inflammatory bowel disease (IBD). Publications from Children’s providers include the below:

• Depression Screening in Pediatric Inflammatory Bowel Disease Clinics: Recommendations and a Toolkit for Implementation

Learn more about ImproveCareNow

The Immunosuppression Withdrawal for Stable Pediatric Liver Transplant Recipients (iWith) clinical trial studied the withdrawal of immunosuppression (anti-rejection) medications in children who have received liver transplants.

Learn the iWith study results

The NASH Clinical Research Network is a NIDDK-sponsored collaborative network that focuses on the causes of, contributing factors to, history of, complications of and therapy for NASH. NASH is caused by a buildup of fat in the liver, which results in inflammation and damage to the liver.

Learn more about the NASH network

View publications from the national collaborative study of infants, children and adolescents with acute liver failure.

• Intravenous N-acetylcysteine in pediatric patients with nonacetaminophen acute liver failure: a placebo-controlled clinical trial
• A Learning Collaborative Approach Increases Specificity of Diagnosis of Acute Liver Failure in Pediatric Patients

SPLIT is a cooperative effort among leading pediatric transplant centers in the U.S. and Canada to advance the science of liver transplants in children.

Large-scale sequencing identifies multiple genes and rare variants associated with Crohn's disease susceptibility.
Sazonovs A, Stevens CR, Venkataraman GR, Yuan K, Avila B, Abreu MT, Ahmad T, Allez M, Ananthakrishnan AN, Atzmon G, Baras A, Barrett JC, Barzilai N, Beaugerie L, Beecham A, Bernstein CN, Bitton A, Bokemeyer B, Chan A, Chung D, Cleynen I, Cosnes J, Cutler DJ, Daly A, Damas OM, Datta LW, Dawany N, Devoto M, Dodge S, Ellinghaus E, Fachal L, Farkkila M, Faubion W, Ferreira M, Franchimont D, Gabriel SB, Ge T, Georges M, Gettler K, Giri M, Glaser B, Goerg S, Goyette P, Graham D, Hämäläinen E, Haritunians T, Heap GA, Hiltunen M, Hoeppner M, Horowitz JE, Irving P, Iyer V, Jalas C, Kelsen J, Khalili H, Kirschner BS, Kontula K, Koskela JT, Kugathasan S, Kupcinskas J, Lamb CA, Laudes M, Lévesque C, Levine AP, Lewis JD, Liefferinckx C, Loescher BS, Louis E, Mansfield J, May S, McCauley JL, Mengesha E, Mni M, Moayyedi P, Moran CJ, Newberry RD, O'Charoen S, Okou DT, Oldenburg B, Ostrer H, Palotie A, Paquette J, Pekow J, Peter I, Pierik MJ, Ponsioen CY, Pontikos N, Prescott N, Pulver AE, Rahmouni S, Rice DL, Saavalainen P, Sands B, Sartor RB, Schiff ER, Schreiber S, Schumm LP, Segal AW, Seksik P, Shawky R, Sheikh SZ, Silverberg MS, Simmons A, Skeiceviciene J, Sokol H, Solomonson M, Somineni H, Sun D, Targan S, Turner D, Uhlig HH, van der Meulen AE, Vermeire S, Verstockt S, Voskuil MD, Winter HS, Young J; Belgium IBD Consortium; Cedars-Sinai IBD; International IBD Genetics Consortium; NIDDK IBD Genetics Consortium; NIHR IBD BioResource; Regeneron Genetics Center; SHARE Consortium; SPARC IBD Network; UK IBD Genetics Consortium, Duerr RH, Franke A, Brant SR, Cho J, Weersma RK, Parkes M, Xavier RJ, Rivas MA, Rioux JD, McGovern DPB, Huang H, Anderson CA, Daly MJ. Nat Genet. 2022 Sep;54(9):1275-1283.  PMID: 36038634

Toward Better Understanding of Pediatric Feeding Disorder: A Proposed Framework for Patient Characterization.
Sharp WG, Silverman A, Arvedson JC, Bandstra NF, Clawson E, Berry RC, McElhanon BO, Kozlowski AM, Katz M, Volkert VM, Goday PS, Lukens CT. J Pediatr Gastroenterol Nutr. 2022 Sep 1;75(3):351-355. PMID: 35687655

Multicenter Cohort Study of Infliximab Pharmacokinetics and Therapy Response in Pediatric Acute Severe Ulcerative Colitis.
Whaley KG, Xiong Y, Karns R, Hyams JS, Kugathasan S, Boyle BM, Walters TD, Kelsen J, LeLeiko N, Shapiro J, Waddell A, Fox S, Bezold R, Bruns S, Widing R, Haberman Y, Collins MH, Mizuno T, Minar P, D'Haens GR, Denson LA, Vinks AA, Rosen MJ. Clin Gastroenterol Hepatol. 2022 Aug 27:S1542-3565(22)00812-6. PMID: 36031093

Eicosatetraynoic Acid and Butyrate Regulate Human Intestinal Organoid Mitochondrial and Extracellular Matrix Pathways Implicated in Crohn's Disease Strictures.
Jurickova I, Bonkowski E, Angerman E, Novak E, Huron A, Akers G, Iwasawa K, Braun T, Hadar R, Hooker M, Han S, Cutler DJ, Okou DT, Kugathasan S, Jegga A, Wells J, Takebe T, Mollen KP, Haberman Y, Denson LA. Inflamm Bowel Dis. 2022 Jul 1;28(7):988-1003.  PMID: 35259271

Targeted Assessment of Mucosal Immune Gene Expression Predicts Clinical Outcomes in Children with Ulcerative Colitis.
Clarkston K, Karns R, Jegga AG, Sharma M, Fox S, Ojo BA, Minar P, Walters TD, Griffiths AM, Mack DR, Boyle B, LeLeiko NS, Markowitz J, Rosh JR, Patel AS, Shah S, Baldassano RN, Pfefferkorn M, Sauer C, Kugathasan S, Haberman Y, Hyams JS, Denson LA, Rosen MJ.  J Crohns Colitis. 2022 Jun 4:jjac075. PMID: 35665804

Parenting Stress over the First Year of Inflammatory Bowel Disease Diagnosis.
Rea KE, Cushman GK, Westbrook AL, Reed B. J Pediatr Psychol. 2022 Jun 4:jsac050. doi: 10.1093/jpepsy/jsac050. Online ahead of print. PMID: 35665814

Methylation quantitative trait loci are largely consistent across disease states in Crohn's disease.
Venkateswaran S, Somineni HK, Kilaru V, Katrinli S, Prince J, Okou DT, Hyams JS, Denson LA, Kellermayer R, Gibson G, Cutler DJ, Smith AK, Kugathasan S, Conneely KN. G3 (Bethesda). 2022 Apr 4;12(4):jkac041. PMID: 35172000 

Use of Patient-Reported Outcomes Measurement Information System Pediatric Measures as Clinical Trial Endpoints: Experience from a Multicenter Pragmatic Trial in Children with Crohn's Disease.
Miller TL, Schuchard J, Carle AC, Forrest CB, Kappelman MD; COMBINE Study Group. J Pediatr. 2022 Mar;242:86-92.e3.  PMID: 34740588 Clinical Trial.

Single-Center Retrospective Review of the Presentation and Initial Care of Esophageal Button Battery Impactions 2007-2020.
Sinclair EM, Agarwal M, Santore MT, Sauer CG, Riedesel EL. Pediatr Emerg Care. 2022 Mar 29:10.1097/PEC.0000000000002690.  PMID: 35353766

Clinical and Host Biological Factors Predict Colectomy Risk in Children Newly Diagnosed With Ulcerative Colitis.
Hyams JS, Brimacombe M, Haberman Y, Walters T, Gibson G, Mo A, Mack D, Griffiths A, Boyle B, LeLeiko N, Markowitz J, Rosh J, Patel A, Shah S, Baldassano R, Pfefferkorn M, Sauer C, Dailey J, Venkateswaran S, Kugathasan S, Denson LA. Inflamm Bowel Dis. 2022 Feb 1;28(2):151-160.  PMID: 33904583 

Evolving Clinical Care in Esophageal Button Batteries: Impact of Expert-Opinion Guideline Adoption and Continued Gaps in Care.
Sinclair EM, Santore MT, Agarwal M, Kitzman J, Sauer CG, Riedesel EL.
J Pediatr Gastroenterol Nutr. 2022 Feb 1;74(2):236-243. doi: 10.1097/MPG.0000000000003346. PMID: 34724451

Early Change in Fecal Calprotectin Predicts One-Year Outcome in Children Newly Diagnosed With Ulcerative Colitis.
Krishnakumar C, Ananthakrishnan AN, Boyle BM, Griffiths AM, LeLeiko NS, Mack DR, Markowitz JF, Rosh JR, Sauer CG, Walters TD, Bonkowski E, Denson LA, Hyams JS, Kugathasan S.
J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):72-78. PMID: 34433783

Assessing the Prevalence of and Risk Factors for Disordered Eating Attitudes and Behaviors in Adolescents With Inflammatory Bowel Disease.
David JG, Chute D, Reed B, Saeed S, DeMatteo D, Atay O, Maddux M, Daly B. Inflamm Bowel Dis. 2022 Jan 5;28(1):143-145.  PMID: 34106253 

Whole-genome sequencing of African Americans implicates differential genetic architecture in inflammatory bowel disease. The American Journal of Human Genetics. 2021.

• Hari K. Somineni, Sini Nagpal, Suresh Venkateswaran, David J. Cutler, David T. Okou, Talin Haritunians, Claire L. Simpson, Ferdouse Begum, Lisa W. Datta, Antonio J. Quiros, Jenifer Seminerio, Emebet Mengesha, Jonathan S. Alexander, Robert N. Baldassano, Sharon Dudley-Brown, Raymond K. Cross, Themistocles Dassopoulos, Lee A. Denson, Tanvi A. Dhere, Heba Iskandar, Gerald W. Dryden, Jason K. Hou, Sunny Z. Hussain, Jeffrey S. Hyams, Kim L. Isaacs, Howard Kader, Michael D. Kappelman, Jeffry Katz, Richard Kellermayer, JohnF .Kuemmerle, Mark Lazarev, Ellen Li, Peter Mannon, Dedrick E. Moulton, Rodney D. Newberry, Ashish S. Patel, Joel Pekow, Shehzad A. Saeed, John F. Valentine, Ming-His Wang, Jacob L. McCauley, Maria T. Abreu, Traci Jester, Zarela Molle-Rios, Sirish Palle, Ellen J. Scherl, John Kwon, John D. Rioux, Richard H. Duerr, Mark S. Silverberg, Michael E. Zwick, Christine Stevens, Mark J. Daly, Judy H. Cho, Greg Gibson, Dermot P.B. McGovern, Steven R. Brant, Subra Kugathasan
• Whether or not populations diverge with respect to the genetic contribution to risk of specific complex diseases is relevant to understanding the evolution of susceptibility and origins of health disparities. Here, we describe a large-scale whole-genome sequencing study of inflammatory bowel disease encompassing 1,774 affected individuals and 1,644 healthy control Americans with African ancestry (African Americans).

Evolving Clinical Care in Esophageal Button Batteries: Impact of Expert-Opinion Guideline Adoption and Continued Gaps in Care. J Pediatr Gastroenterol Nutr. 2021.

• Elizabeth M Sinclair, Matthew T Santore, Maneesha Agarwal, Jamie Kitzman, Cary G Sauer, Erica L Riedesel
• Esophageal button battery impactions (BBI) in children pose a significant danger to children. With this study, we aimed to describe the care of these patients prior to and following adoption of guidelines at a single center.

Creation of a Pediatric Choledocholithiasis Prediction Model. J Pediatr Gastroenterol Nutr. 2021.

• Reuven Zev Cohen, Hongzhen Tian, Cary G Sauer, Field F Willingham, Matthew T Santore, Yajun Mei, A Jay Freeman
• Alanine aminotransferase (ALT), total bilirubin, alkaline phosphatase, and common bile duct diameter via ultrasound were found to be the key clinical variables to determine the likelihood of choledocholithiasis.

Early Change in Fecal Calprotectin Predicts One-Year Outcome in Children Newly Diagnosed with Ulcerative Colitis. J Pediatr Gastroenterol Nutr. 2021

• Krishnakumar C, Ananthakrishnan AN, Boyle BM, Griffiths AM, LeLeiko NS, Mack DR, Markowitz JF, Rosh JR, Sauer CG, Walters TD, Bonkowski E, Denson LA, Hyams JS, Kugathasan S
• While fecal calprotectin (FC) is used to assess disease activity in ulcerative colitis (UC) there are little data concerning the role of serial FC levels at diagnosis in predicting clinical course. We sought to determine whether FC at diagnosis or early change following therapy predicts clinical outcome in pediatric UC.methods.

Clinical and Host Biological Factors Predict Colectomy Risk in Children Newly Diagnosed With Ulcerative Colitis. Inflamm Bowel Dis. 2021.

• Hyams JS, Brimacombe M, Haberman Y, Walters T, Gibson G, Mo A, Mack D, Griffiths A, Boyle B, LeLeiko N, Markowitz J, Rosh J, Patel A, Shah S, Baldassano R, Pfefferkorn M, Sauer C, Dailey J, Venkateswaran S, Kugathasan S, Denson LA.
• This was a multicenter inception cohort study of children (ages 4-17 years) newly diagnosed with UC treated with standardized initial regimens of mesalamine or corticosteroids (CS) depending upon initial disease severity.

Quality Improvement Methodology Optimizes Infliximab Levels in Pediatric Patients with Inflammatory Bowel Disease. Pediatr Qual Saf. 2021.

• Jennifer Hellmann, Renee K Etter, Lee A Denson, Phillip Minar, Denise Hill, Dana Dykes, Michael J Rosen
• Quality improvement methodology was effective in improving provider adherence to infliximab therapeutic drug monitoring guidelines. Improvement in adherence to guidelines directly improved the percentage of patients achieving target infliximab levels at any time during infliximab therapy.

Gastrointestinal Factors Associated With Hospitalization in Infants With Cystic Fibrosis: Results From the Baby Observational and Nutrition Study. J Pediatr Gastroenterol Nutr. 2021.

• Sathe M, Huang R, Heltshe S, Eng A, Borenstein E, Miller SI, Hoffman L, Gelfond D, Leung DH, Borowitz D, Ramsey B, Freeman AJ
• Factors associated with a higher risk for GI-related admission during the first 12 months include high PERT dosing, exposure to acid suppressive medications, higher fCP levels, and/or relative abundance of fecal K pneumoniae early in life.

Designing the GALAXY Study: Partnering with the Cystic Fibrosis Community to Optimize Assessment of Gastrointestinal Symptoms. J Cyst Fibros. 2021.

• Freeman AJ, Sathe M, Aliaj E, Borowitz D, Fogarty B, Goss CH, Freedman S, Heltshe SL, Khan U, Riva D, Roman C, Romasco M, Schwarzenberg SJ, Ufret-Vincenty CA, Moshiree B
• GI involvement among persons with CF is highly prevalent, representing a significant source of morbidity. Persons with CF have identified GI concerns as a top research priority, yet universal clinical outcome measures capturing many of the GI symptoms experienced in CF are lacking. The GALAXY study was envisioned to address this unmet need.

Serum Bile Acids as a New Prognostic Biomarker in Biliary Atresia Following Kasai Portoenterostomy.
Harpavat S, Hawthorne K, Setchell KDR, Narvaez Rivas M, Henn L, Beil CA, Karpen SJ, Ng VL, Alonso EM, Bezerra JA, Guthery SL, Horslen S, Loomes KM, McKiernan P, Magee JC, Merion RM, Molleston JP, Rosenthal P, Thompson RJ, Wang KS, Sokol RJ, Shneider BL; Childhood Liver Disease Research Network (ChiLDReN).
Hepatology. 2022 Sep 21. PMID: 36131538

Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia.
Bass LM, Ye W, Hawthorne K, Leung DH, Murray KF, Molleston JP, Romero R, Karpen S, Rosenthal P, Loomes KM, Wang KS, Squires RH, Miethke A, Ng VL, Horslen S, Kyle Jensen M, Sokol RJ, Magee JC, Shneider BL; ChiLDReN. Hepatology. 2022 Sep;76(3):712-726.  PMID: 35271743

Serum Biomarkers Correlated with Liver Stiffness Assessed in a Multi-center Study of Pediatric Cholestatic Liver Disease.
Leung DH, Devaraj S, Goodrich NP, Chen X, Rajapakshe D, Ye W, Andreev V, Minard CG, Guffey D, Molleston JP, Bass LM, Karpen SJ, Kamath BM, Wang KS, Sundaram SS, Rosenthal P, McKiernan P, Loomes KM, Jensen MK, Horslen S, Bezerra JA, Magee JC, Merion RM, Sokol RJ, Shneider BL; For ChiLDReN. Hepatology. 2022 Sep 7. PMID: 36069569

Ileal bile acid transporter inhibition in Cyp2c70 KO mice ameliorates cholestatic liver injury.
Truong JK, Bennett AL, Klindt C, Donepudi AC, Malla SR, Pachura KJ, Zaufel A, Moustafa T, Dawson PA, Karpen SJ.
J Lipid Res. 2022 Sep;63(9):100261. PMID: 35934110

Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial.
Thompson RJ, Arnell H, Artan R, Baumann U, Calvo PL, Czubkowski P, Dalgic B, D'Antiga L, Durmaz Ö, Fischler B, Gonzalès E, Grammatikopoulos T, Gupte G, Hardikar W, Houwen RHJ, Kamath BM, Karpen SJ, Kjems L, Lacaille F, Lachaux A, Lainka E, Mack CL, Mattsson JP, McKiernan P, Özen H, Rajwal SR, Roquelaure B, Shagrani M, Shteyer E, Soufi N, Sturm E, Tessier ME, Verkade HJ, Horn P.  Lancet Gastroenterol Hepatol. 2022 Sep;7(9):830-842. PMID: 35780807 

Post hoc, ergo propter hoc? Evaluating a Potential Increase in Childhood Severe Hepatitis in a Post-COVID World.
Gupta NA, Romero R. J Pediatric Infect Dis Soc. 2022 Aug 30;11(8):354-356. PMID: 35674749 

Natural History of Liver Disease in a Large International Cohort of Children with Alagille syndrome: Results from The GALA Study.
Vandriel SM, Li LT, She H, Wang JS, Gilbert MA, Jankowska I, Czubkowski P, Gliwicz-Miedzińska D, Gonzales EM, Jacquemin E, Bouligand J, Spinner NB, Loomes KM, Piccoli DA, D'Antiga L, Nicastro E, Sokal É, Demaret T, Ebel NH, Feinstein JA, Fawaz R, Nastasio S, Lacaille F, Debray D, Arnell H, Fischler B, Siew S, Stormon M, Karpen SJ, Romero R, Kim KM, Baek WY, Hardikar W, Shankar S, Roberts AJ, Evans HM, Jensen MK, Kavan M, Sundaram SS, Chaidez A, Karthikeyan P, Sanchez MC, Cavalieri ML, Verkade HJ, Lee WS, Squires JE, Hajinicolaou C, Lertudomphonwanit C, Fischer RT, Larson-Nath C, Mozer-Glassberg Y, Arikan C, Lin HC, Quintero Bernabeu J, Alam S, Kelly D, Carvalho E, Ferreira CT, Indolfi G, Quiros-Tejeira RE, Bulut P, Calvo PL, Önal Z, Valentino PL, Desai DM, Eshun J, Rogalidou M, Dezsőfi A, Wiecek S, Nebbia G, Borges Pinto R, Wolters VM, Tamara ML, Zizzo AN, Garcia J, Schwarz K, Beretta M, Sandahl TD, Jimenez-Rivera C, Kerkar N, Brecelj J, Mujawar Q, Rock N, Busoms CM, Karnsakul W, Lurz E, Santos-Silva E, Blondet N, Bujanda L, Shah U, Thompson RJ, Hansen BE, Kamath BM; Global ALagille Alliance (GALA) Study Group. Hepatology. 2022 Aug 29. PMID: 36036223 

Use of funded multicenter prospective longitudinal databases to inform clinical trials in rare diseases-Examination of cholestatic liver disease in Alagille syndrome.
Shneider BL, Kamath BM, Magee JC, Goodrich NP, Loomes KM, Ye W, Spino C, Alonso EM, Molleston JP, Bezerra JA, Wang KS, Karpen SJ, Horslen SP, Guthery SL, Rosenthal P, Squires RH, Sokol RJ; Childhood Liver Disease Research Network (ChiLDReN). Hepatol Commun. 2022 Aug;6(8):1910-1921. PMID: 35506349 

Randomized placebo-controlled trial of losartan for pediatric NAFLD.
Vos MB, Van Natta ML, Blondet NM, Dasarathy S, Fishbein M, Hertel P, Jain AK, Karpen SJ, Lavine JE, Mohammad S, Miriel LA, Molleston JP, Mouzaki M, Sanyal A, Sharkey EP, Schwimmer JB, Tonascia J, Wilson LA, Xanthakos SA; NASH Clinical Research Network. Hepatology. 2022 Aug;76(2):429-444. PMID: 35133671 Clinical Trial.

Impact of long-term administration of maralixibat on children with cholestasis secondary to Alagille syndrome.
Shneider BL, Spino CA, Kamath BM, Magee JC, Ignacio RV, Huang S, Horslen SP, Molleston JP, Miethke AG, Kohli R, Leung DH, Jensen MK, Loomes KM, Karpen SJ, Mack C, Rosenthal P, Squires RH, Baker A, Rajwal S, Kelly D, Sokol RJ, Thompson RJ; for ChiLDReN and UK IMAGO/IMAGINE Investigators. Hepatol Commun. 2022 Aug;6(8):1922-1933.  PMID: 35672955 

Recent Insights into Pediatric Primary Sclerosing Cholangitis.
Stevens JP, Gupta NA. Clin Liver Dis. 2022 Aug;26(3):489-519.  Epub 2022 Jun 25. PMID: 35868687

Fat Soluble Vitamin Assessment and Supplementation in Cholestasis.
Kamath BM, Alonso EM, Heubi JE, Karpen SJ, Sundaram SS, Shneider BL, Sokol RJ. Clin Liver Dis. 2022 Aug;26(3):537-553. PMID: 35868689 Review.

Acute Hepatitis in Children in 2022 - Human Adenovirus 41?
Karpen SJ. N Engl J Med. 2022 Aug 18;387(7):656-657.  PMID: 35830650 No abstract available.

The prevalence of alcoholic and nonalcoholic fatty liver disease in adolescents and young adults in the United States: analysis of the NHANES database.
Alkhouri N, Almomani A, Le P, Payne JY, Asaad I, Sakkal C, Vos M, Noureddin M, Kumar P. BMC Gastroenterol. 2022 Jul 30;22(1):366.  PMID: 35907827 

Portal vein complications and outcomes following pediatric liver transplantation: Data from the Society of Pediatric Liver Transplantation.
Stevens JP, Xiang Y, Leong T, Naik K, Gupta NA. Liver Transpl. 2022 Jul;28(7):1196-1206. PMID: 35092344

Association of Prenatal Exposure to Endocrine-Disrupting Chemicals With Liver Injury in Children.
Midya V, Colicino E, Conti DV, Berhane K, Garcia E, Stratakis N, Andrusaityte S, Basagaña X, Casas M, Fossati S, Gražuleviciene R, Haug LS, Heude B, Maitre L, McEachan R, Papadopoulou E, Roumeliotaki T, Philippat C, Thomsen C, Urquiza J, Vafeiadi M, Varo N, Vos MB, Wright J, McConnell R, Vrijheid M, Chatzi L, Valvi D.
JAMA Netw Open. 2022 Jul 1;5(7):e2220176. PMID: 35793087 

Education and Psychosocial Factors Predict Odds of Death After Transfer to Adult Healthcare in Pediatric Liver Transplant Patients.
Stevens JP, Gillespie S, Hall L, Tisheh J, Ford R, Gupta NA. J Pediatr Gastroenterol Nutr. 2022 Jun 27. PMID: 35767565

Cholestatic liver diseases of genetic etiology: Advances and controversies.
Ibrahim SH, Kamath BM, Loomes KM, Karpen SJ. Hepatology. 2022 Jun;75(6):1627-1646.  PMID: 35229330 

Incidence of Type 2 Diabetes in Children With Nonalcoholic Fatty Liver Disease.
Newton KP, Wilson LA, Crimmins NA, Fishbein MH, Molleston JP, Xanthakos SA, Behling C, Schwimmer JB; Nonalcoholic Steatohepatitis Clinical Research Network.
Clin Gastroenterol Hepatol. 2022 Jun 13:S1542-3565(22)00556-0.  PMID: 35709934 Free article.

Nonalcoholic fatty liver disease risk and histologic severity are associated with genetic polymorphisms in children.
Goyal NP, Rosenthal SB, Nasamran C, Behling CA, Angeles JE, Fishbein MH, Harlow KE, Jain AK, Molleston JP, Newton KP, Ugalde-Nicalo P, Xanthankos SA, Yates K, Schork NJ, Fisch KM, Schwimmer JB; NASH Clinical Research Network. Hepatology. 2022 May 13.  PMID: 35560106

Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver.
Leung DH, Sorensen LG, Ye W, Hawthorne K, Ng VL, Loomes KM, Fredericks EM, Alonso EM, Heubi JE, Horslen SP, Karpen SJ, Molleston JP, Rosenthal P, Sokol RJ, Squires RH, Wang KS, Kamath BM, Magee JC; Childhood Liver Disease Research Network (ChiLDReN). J Pediatr Gastroenterol Nutr. 2022 Jan 1;74(1):96-103. PMID: 34694263 

Factors associated with improved patient and graft survival beyond 1 year in pediatric liver transplantation.
Squires JE, Bilhartz J, Soltys K, Hafberg E, Mazariegos GV, Gupta NA, Anand R, Anderson SG, Miloh T; Society of Pediatric Liver Transplantation. Liver Transpl. 2022 May 12. PMID: 35555876

Predictors of survival following liver transplantation for pediatric hepatoblastoma and hepatocellular carcinoma: Experience from the Society of Pediatric Liver Transplantation (SPLIT).
Boster JM, Superina R, Mazariegos GV, Tiao GM, Roach JP, Lovell MA, Greffe BS, Yanni G, Leung DH, Elisofon SA, McDiarmid SV, Gupta NA, Lobritto SJ, Lemoine C, Stoll JM, Vitola BE, Daniel JF, Sayed BA, Desai DM, Martin AE, Amin A, Anand R, Anderson SG, Sundaram SS. Am J Transplant. 2022 May;22(5):1396-1408. PMID: 34990053

North American Biliary Stricture Management Strategies in Children After Liver Transplantation: A Multicenter Analysis From the Society of Pediatric Liver Transplantation (SPLIT) Registry.
Valentino PL, Wang T, Shabanova V, Ng VL, Bucuvalas JC, Feldman AG, Gonzalez-Peralta RP, Gupta NA, Miloh TA, Mohammad S, Pace E, Sundaram SS, Yazigi NA, Soltys K; Society of Pediatric Liver Transplantation (SPLIT). Liver Transpl. 2022 May;28(5):819-833. PMID: 34837468

Variation in Alanine Aminotransferase in Children with Non-Alcoholic Fatty Liver Disease.
Castillo-Leon E, Morris HL, Schoen C, Bilhartz J, McKiernan P, Miloh T, Palle S, Kabbany MN, Munoz B, Mospan AR, Rudolph B, Xanthakos SA, Vos MB, Target-Nash Investigators. Children (Basel). 2022 Mar 8;9(3):374.  PMID: 35327746 

Dietary sugar restriction reduces hepatic de novo lipogenesis in adolescent boys with fatty liver disease

• Catherine C Cohen, Kelvin W Li, Adina L Alazraki, Carine Beysen, Carissa A Carrier, Rebecca L Cleeton, Mohamad Dandan, Janet Figueroa, Jack Knight-Scott, Cynthia J Knott, Kimberly P Newton, Edna M Nyangau, Claude B Sirlin, Patricia A Ugalde-Nicalo, Jean A Welsh, Marc K Hellerstein, Jeffrey B Schwimmer, Miriam B Vos
• Hepatic de novo lipogenesis (DNL) is elevated in nonalcoholic fatty liver disease (NAFLD). Improvements in hepatic fat by dietary sugar reduction may be mediated by reduced DNL, but data are limited, especially in children. We examined the effects of 8 weeks of dietary sugar restriction on hepatic DNL in adolescents with NAFLD and correlations between DNL and other metabolic outcomes.

PRO-C3, a Serological Marker of Fibrosis, During Childhood and Correlations With Fibrosis in Pediatric NAFLD. Hepatol Commun. 2021.

• Cohen CC, Castillo-Leon E, Farris AB, Caltharp SA, Cleeton RL, Sinclair EM, Shevell DE, Karsdal MA, Nielsen MJF, Leeming DJ, Vos MB
• The objectives of this study were to examine PRO-C3 (a neo-epitope pro-peptide of type III collagen formation) levels across childhood/adolescence and associations with advanced fibrosis in pediatric NAFLD. 

Patient Determinants for Histologic Diagnosis of NAFLD in the Real World: A TARGET-NASH Study. Hepatol Commun. 2021.

• Barritt AS, Watkins S, Gitlin N, Klein S, Lok AS, Loomba R, Schoen C, Reddy KR, Trinh HN, Mospan AR, Vos MB, et al.
• The aim of this study was to determine patient factors predictive of histologic versus empiric clinical diagnosis of NAFLD in real-world practice.

Neurodevelopmental Outcomes in Children with Inherited Liver Disease and Native Liver. J Pediatr Gastroenterol Nutr. 2021.

• Leung DH, Sorensen LG, Ye W, Hawthorne K, Ng VL, Loomes KM, Fredericks EM, Alonso EM, Heubi JE, Horslen SP, Karpen SJ, et al.
• To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment.

Medical Management of Chronic Pancreatitis in Children: A Position Paper by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Pancreas Committee. Journal of Pediatric Gastroenterology and Nutrition.

• Freeman, A. Jay, et al.
• This position paper summarizes the current understanding of the medical management of chronic pancreatitis (CP) in children in light of the existing medical literature, incorporating recent advances in understanding of nutrition, pain, lifestyle considerations, and sequelae of CP.

Pancreatitis in Children. Pediatr Clin North Am. 2021.

• Reuven Zev Cohen, A Jay Freeman
• Pediatric pancreatitis describes a spectrum covering acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis, each with varying clinical manifestations and risk factors requiring a tailored diagnostic approach. 

Order Set Use and Education Association With Pediatric Acute Pancreatitis Outcomes. Hosp Pediatr. 2021.

• Shah M, Leong T, Freeman AJ.
• Pediatric-specific recommendations for treatment of AP were recently developed by North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, but their impact on clinical outcomes has yet to be evaluated. We developed an AP order set on the basis of these recommendations to assess impact on clinical outcomes.

SARS-CoV2 Infection in Children with Liver Transplant and Native Liver Disease. J Pediatr Gastroenterol Nutr. 2021

• Mohit Kehar, Noelle H Ebel, Vicky L Ng, Jairo Eduardo Rivera Baquero, Daniel H Leung, Voytek Slowik, Nadia Ovchinsky, Amit A Shah, Ronen Arnon, Tamir Miloh, Nitika Gupta, Saeed Mohammad, Debora Kogan-Liberman, James E Squires, Maria Camila Sanchez, Amber Hildreth, Linda Book, Christopher Chu, Leina Alrabadi, Ruba Azzam, Bhavika Chepuri, Scott Elisofon, Rachel Falik, Lisa Gallagher, Howard Kader, Douglas Mogul, Quais Mujawar, Shweta S Namjoshi, Pamela L Valentino, Bernadette Vitola, Nadia Waheed, Ming-Hua Zheng, Steven Lobritto, Mercedes Martinez
• Increased mortality risk because of severe acute respiratory syndrome coronavirus-2 (SARS-CoV2) infection in adults with native liver disease (LD) and liver transplant (LT) is associated with advanced age and comorbid conditions. We aim to report outcomes for children with LD and LT enrolled in the NASPGHAN/SPLIT SARS-CoV2 registry.

North American biliary stricture management strategies in children post liver transplant: multicenter analysis from the SPLIT Registry. Liver Transpl. 2021.

• Valentino PL, Wang T, Shabanova V, Ng VL, Bucuvalas JC, Feldman AG, Gonzalez-Peralta RP, Gupta NA, et al.
• Study objectives included analyses of outcomes associated with biliary stricture management strategies via PTC, ERCP or surgery.

Presentation and Outcomes of Infants With Idiopathic Cholestasis: A Multicenter Prospective Study. J Pediatr Gastroenterol Nutr. 2021.

• Hertel PM, Hawthorne K, Kim S, Finegold MJ, Shneider BL, Squires JE, Gupta NA, et al.
• The aim of the study was to determine the frequency and natural history of infantile idiopathic cholestasis (IC) in a large, prospective, multicenter cohort of infants.

Resolution of recurrent pediatric acute liver failure with liver transplantation in a patient with NBAS mutation. Pediatr Transplant. 2021.

• Duke Geem, Wenxiao Jiang, Heather B Rytting, Shanmuganathan Chandrakasan, Anand Salem, James P Stevens, Saul J Karpen, Joseph F Magliocca, Rene Romero, Dellys Soler Rodriguez
• A growing body of research has identified mutations in the NBAS gene to be associated with recurrent acute liver failure and multi-systemic disease including short stature, skeletal dysplasia, facial dysmorphism, immunologic abnormalities, and Pelger-Huët anomaly.

Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure. J Pediatr. 2021.

• Rodriguez DS, Mao C, Mahle WT, Kanter KR, Alazraki A, Braithwaite K, Rytting H, Caltharp S Magliocca JF, Romero R
• To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation.